Category Archives: Neurology

Thank you to Andrew for presenting an interesting case of a young women with ESRD 2/2 lupus nephritis and failed transplant on PD presenting with painful purpura c/f calcinosis cutis. The patient subsequently developed AMS, sinus tachycardia, clonus and hyperreflexia c/f serotonin syndrome.

Calcinosis Cutis

Calcinosis cutis is described as the deposition of insoluble calcium salts in the skin and subcutaneous tissue.

There are five subtypes of calcinosis cutis: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.

There is an association with autoimmune disorders – mostly dermatomyositis and mixed connective tissue disorders. It is less commonly associated with SLE.

Associated with trauma, underlying Calcium and Phosphorus metabolism disorders: the higher the Ca++ and Phos the higher the risk (one rule of thumb is Ca++xPhos > 55 puts this patient at high risk)

NEJM

Calciphylaxis

Calciphylaxis is a rare and serious disorder that presents with skin ischemia and necrosis and is characterized histologically by calcification of dermal arterioles

Main risk factor is ESRD and is more formally know as calcific uremic arteriolopathy (CUA)

Morning report today was like a regular primary care clinic day – one daily ditty and two patients all in one hour no problem! Thanks Lily for presenting two awesome cases.

Case 1: A young woman on OCPs with right sided facial numbness and facial droop sparing the forehead found to have MS.

Unilateral Facial Paralysis:

The key to evaluation is determining if the lesion is central vs. peripheral

The forehead is spared in a central process due to bilateral innervation

There are possible peripheral causes that spare the forehead BUT forehead sparing should raise your suspicion for a central process

Pearl from Jody is that forehead weakness can be subtle as patients try to overcome it with muscles of the scalp. In addition to having the patient raise their eyebrows, have them maintain raised eyebrows against your force.

Central

Stroke

Malignancy

Demyelinating diseases

Migraines

Peripheral – Facial nerve palsy

Infections: Herpes zoster, otitis media, lyme, HIV

Immune-mediated: GBS

Autoimmune: sarcoid, Sjogrens

Idiopathic (Bell’s palsy)

Case 2: A 45 yo G1P0 female with recent spontaneous abortion and RUE DVT with chronic right arm pain and skin changes found to have complex regional pain syndrome.

We reviewed indications for thrombophilia work-up.

We should not test every individual with an unprovoked VTE because for most people it will not change management.

Who does it change management in?

Women planning conception

Possible need to treatment with pro-thrombotic agents such as estrogen

In general we should consider testing in the following populations:

Young patients (<50) with weak provoking factors or strong family history

Thank you to Nadine for presenting a case of a young woman with very complex medical history including cryptogenic cirrhosis s/p TIPS, hepatic encephalopathy on lactulose and rifaximin listed for transplant, aplastic anemia, CAD s/p mid-LAD thrombectomy, LAD dissection, APS antibody positive on ASA, short telomere syndrome, who was admitted for neutropenic fever and evolution of spontaneous subdural hematomas in the setting of coagulopathy and thrombocytopenia. Wow!

Neutropenic Fever:

ANC <500 or <1000 with anticipated decline

Single temperature >38.3 or >38 sustained for more than 1 hour

When you think about empiric coverage, consider patients as either high risk or low risk. Here’s a breakdown of high vs. low from Medscape.

Most of the guidelines for neutropenia come from patients with chemotherapy induced neutropenia – these patients are at the highest risk for acute infection due to the combination of both low neutrophil count and mucositis.

However, patients with other causes of neutropenia who have had absolute low neutrophils for prolonged periods of time, may be at higher risk for indolent infections.

Infected Subdural Hematomas

As HH mentioned most subdural infections represent local extension of paranasal sinusitis or otitis, or are complication of intracranial surgery.

Infection of a subdural hematoma is a usual cause of subdural empyema with < 50 cases reported (see article below).

May transform in the setting of pre-existing subdural via hematogenous infection (concerning for our patient with new murmur and AMS).

Signs and symptoms are non-specific and include altered sensorium (depressed level of consciousness), fevers and focal deficits.

MRI has become the imaging modality of choice in patients with infected subdural hematoma. It is superior to CT scans for the demonstration of extra axial fluid and rim enhancement, and in the visualization of the presence of pus.

Definitive management is surgical: both burr hole and craniotomy.

The best surgical option is not well defined, however based on limited data the recurrence rate seems to be lower with craniotomy.

Thanks Amanda for sharing your case of a middle aged male with a past history of diabetes and HTN presenting with diplopia found to have a sixth nerve palsy due to a pontine infarct.

Diplopia Pearls

Your approach to diplopia should be the same as we approach any neurologic deficit – Localize the lesion along the anatomic pathway

Central

Peripheral cranial nerve

Both a mononeuropathy or external compression of the cranial nerve

Extraocular muscles

neuromuscular junction

Globe (retina, cornea, lens)

Monocular diplopia vs. binocular diplopia

Binocular diplopia: If the diplopia is present only when both eyes are open then the diplopia is due to ocular misalignment

Monocular: Most often due to diseasse of the cornea or lens, less commonly the retina or cerebral lesion

Extraoccular muscle weakness can be subtle. LT pearl: when testing EOM, move your finger fast as this will be more likely to demonstrate subtle weakness than slow movements.

Cranial nerve palsies

3rd nerve: longest cranial nerve after exiting the brainstem, more commonly affected by compression from an aneurysm or tumor. When a third nerve palsy is due to microinfarction from diabetes it typically spares the pupil. Therefore pupillary defects should raise your suspicion for intracranial lesion

4th nerve: Most prone to injury from trauma and more rarely affected by microinfarction

Morning, some good neuro critical care action at the VA this week, thought we’d cover something today not yet covered on the blog: non-invasive estimations of elevated ICP, namely fundoscopy and ocular ultrasound

Dan Reiss correctly pointed out the acute finding in elevated ICP on an ocular ultrasound is the optic nerve sheath diameter, a handful of smaller studies (n in each about 40-60) have evaluated the correlation between nerve sheath diameter and MRI/CT findings of elevated CT, Sensitivity and Specificy depends on the cutoff chosen, in adults typically a ONSD of >4.5mm is considered too high, which yields a sens and spec of around 70-80% for the detection of elevated ICP. The challenge here, as with fundoscopy, is inter-observer variability presumably due to operator characteristics. Here is a review paper on non-invasive determination of ICP.

Other quick pearls

#generally opt for beta blockers and ccb in the acute treatment of hypertensive emergency due to the theoretical concern for cerebral vasodilation with nitro/hydral, nicardipine and esmolol are good options

#PRES, covered elswhere, stands for posterior reversible encephalopathy syndrome, though not always posterior or reversible. Remember, MRI is the diagnostic test for PRES.

At the General, we give you a little bit of this and a little bit of that in report. And same thing goes for the chiefs’ blog. This is a quick run-through of a few recent legendary reports!

In Neuro Report today, we crushed, I mean discussed, hypercarbic respiratory failure and the role of neuromuscular causes for it. We were joined by neurology guru, Andy Romeo, and here are a few of his pearls:

-Whenever you come across someone reporting dysphagia, make sure to ask about other bulbar sx’s
-In a patient with increased work of breathing in whom you’re considering if diaphragmatic weakness is playing a role, check neck flexor strengthto assess if a new neuromuscular weakness is present
–MIF & VC are the confrontational tests for the diaphragm. To remind ourselves about those two entities:

For Vital capacity (VC) and Mean inspiratory force (MIF), there is the 20-30 rule

VC: deep breath and exhalation maximally into spirometer; goal is at least 20cc/kg

MIF: inhalation against a closed valve with negative force recorded; goal is “more negative” than -30 cmH20. -60cmH20 is expected or what is associated with weak cough in NL person

In a recent ID report, we discussed the well-known penile lesion ddxand added in a lesser known branch point of the *PRURITIC* penile lesion. The following is a non-exhaustive (and likely with much overlap) summary of what we came up with:

*PRURITIC* penile lesion

Fixed drug reaction, DRESS/DHR

Yeast

Infestation-scabies/pubic lice

HSV

Behcet’s

So how do we diagnosis LGV? Does our usual urine test work??????Lisa Winston teaches us:

Turns out the usual Chalmydia culture or the more commonly ordered/sensitive NAAT test will bepositive in LGV as the serovars will be picked up—it just won’t specify that it detected the L1-3 serovars. Usually when the sx’s are classic, empiric tx (longer course) is initiated. If you want a definitive dx, you can talk to colleagues at communicable dz and public health to see if need to send serology or special PCR to the SF public health lab (and then potentially to state’s public health lab or CDC).

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Lastly, Mike and Carinepresented a patient in intern report with MV endocarditis 2/2 MSSA where we discussed the role of early surgical intervention in infective endocarditis.

There is a fantastic 2013 NEJM Infective Endocarditis review article by Hoen and Duval that breaks down the indications for surgery into three big categories: heart failure,uncontrolled infection, and prevention of embolic events. Or in image form:

For those of you who want more…

Punag, one of the cardiology fellows, passes on the following for the ACC/AHA class indications for surgical intervention:

Early surgery is recommended for patients with complicated infective endocarditis (IE), but data from randomized trials are scarce. The following are points to remember about the timing of surgery among patients with IE:

The main indications for early surgery in IE are heart failure, uncontrolled infection, and prevention of embolization. The reduction in mortality with surgery is greatest among patients with IE and moderate to severe heart failure.

Neurological complications. Patients with a neurological complication may have other indications for early surgery. However, early surgery may pose a significant risk for perioperative neurological deterioration (related to anticoagulation potentiating the risk of intracerebral bleeding, and to hypotension during cardiopulmonary bypass aggravating neurological ischemia and edema).

Prosthetic valve IE. Prosthetic valve endocarditis is the most serious form of IE, and more difficult to treat using antibiotics alone. In general, current guidelines support consideration of a surgical strategy for high-risk subgroups with prosthetic valve IE, including patients with heart failure, abscess, or persistent fever.

Definitions of early surgery. There is no consensus as to the optimal timing of early surgery. The ESC guideline classifies surgical indications in IE as emergent (within 24 hours), urgent (within a few days), and elective (after 1-2 weeks of antibiotic therapy). The AHA/ACC guideline defines early surgery as occurring during the initial hospitalization and before completion of a full therapeutic course of antibiotics.

This case hinged upon identifying the patient’s cranial neuropathies and trying to identify an atomic location whether those cranial nerves ran together. So here’s a quick refresher on the facial nerve and the geniculate ganglion.

The facial nerve exits the brain stem, courses through the temporal bone, then forms a knee-shaped ganglion at its exit

As this less detailed image shows. The facial nerve runs *extremely close* to the ear when exits the temporal bone. So when VZV reactivates in the geniculate ganglion, it travels transaxonally through the nerves to skin around the ear, causing vesicles there.

Most VZV reactivation in the face is in the trigeminal nerve, which is why we are so accustomed to seeing zoster in a V1, V2, or V3 distribution. Turns out it can affect whatever nerve it feels like.

When the facial nerve runs next to the ear, it also runs in close quarters with the vestibulocochlear nerve. So Ramsey-Hunt can also include deafness or vestibular symptoms and for this reason is considered an ENT emergency.

Ramsey Hunt is usually managed with acyclovir + steroids. A cochrane review showed no evidence of benefit for acyclovir but the overall evidence quality was poor.

Ramsey Hunt, a peripheral nerve manifestation of zoster, should be distinguished from CNS zoster which can be devastating. Zoster encephalitis, if it affects the brain stem, can also pick off specific cranial nerves. Because our patient also had deficits in non-adjacent cranial nerves (IX and X), we suspected brainstem disease.

Multiple Cranial Neuropathies

So interesting! So serious. More pearls about them here: https://ucsfmed.wordpress.com/2016/10/28/zsfg-neuro-report-multiple-cranial-neuropathies-spotlight-on-the-cavernous-sinus/